[Clinical picture and immunology of Wegener's granulomatosis]

In the last 21 years, 22 histologically proven cases of Wegener's granulomatosis (WG) were observed and treated. In 19 patients WG appeared primarily in the ENT-area. Ten patients had middle ear involvement. Whereas, in 9 cases the inner ear was affected too. Before introduction of immunosuppressive therapy the patients (n = 8) died averagely after 5.6 months after onset of the first symptoms. Under therapy with cyclophosphamide and prednisone total remission of WG can be reached for long times (n = 14, 3.2 years of follow up). In 14 cases serologically anti-cytoplasmatic antibodies against neutrophil granulocytes were determined in high activity stage of WG. In combination with the clinical and histological findings the determination of these antibodies gives the possibility to solve difficulties in differential diagnosis of WG.